Flashcards in Rheumatologic Lung DZ Deck (68)
What are the five rheumatologic diseases that often present with pleuropulmonary symptoms
Systemic sclerosis, systemic lupus rheumatoid arthritis, dermatomyositis, Sjrogen Syndrome
Which of the fiv efeatures the MOST lung dz symptoms
Systemic sclerosis (aka scleroderma)
Lung dz in scleroderma takes two forms, they are:
Interstitial and Vascular
Interstitial lung dz in scleroderma or "SCLERODERMA LUNG" most often presesnts as
- non-specific interstitial pneumonia or interstitial fibrosis
- Concentric arterial thickening
About 67% of systemic sclerosis pts get
interstitial lung dz
About 20% of systemic sclerosis pts suffer from
The pathophysiological mediators in Scleroderma Lung are:
TNF alpha does
IL8= neutrophil attractant and activator
TNFalpha-= neutrophil attractant and promoter of neutrophil endothelial binding
MIF1 alpha does
promoter of neutrophil chemotaxis
T cell recruiter and activator
promoter of fibroplasia
Scleroderma lung favors which part of the lung?
Base, posterior, periphery.
You will first see Ground glass appearance on the CT then will become more reticular
Early histology of scleroderma lung shows
Non-specific interstitial pneumonia with lymphocytes and macrophages followed by fibroblasts and collagen
Trichrome stain stains collagen what color?
The vascular disease component of scleroderma lung causes
pulmonary hypertension and cor pulmonaale
What is CREST syndrome
When the the vascular component of scleroderma appears without the interstitial lung disease.
It is associated with anti-centromere antibodies and limited systemic sclerosis
What are the symptoms of SS lung dz
Dyspnea and dry cough due to increased work load of breathing due to fibrosis
Signs of SS Lung
Crackles due to the sudden opening of small airways abnormally closed by the pressure of interstitial inflammation
SS Lung dz diagnosis
Associtation of symptoms , signs, and radiology of interstitial lung dz with skin, esophageal, and renal manifestations of scleroderma.
- Anti-scl70 (DNA Topoisomerase) antibodies
Treatment of systemic sclerosis
Why don't you treat SS lung dz with steroids?
Renal crisis has been reported
Most common cause of death for scleroderma pts
Prognosis of scleroderma pts from initial survival?
Pleuropulmonary disease of lupus generally has 5 manifestations. THey are:
- Acute Lupus pneumonitis
- Nonspecific interstitial pneumonia
- Pulmonary Vascular dz
- Shrinking lung syndrome
The most common pleuropulmonary manifestation of lupus by a long shot is:
Pleuritis (present in 20% at onset, 50% over time, and 100% at autopsy)
Lupus pleuritis is typically
Is fibrinous pleuritis specific for lupus>
Pleural effusions in lupus are typically
small and bilateral